Pediatrics International最新文献

Background: The appropriate reference value of the urinary protein-to-creatinine ratio (PCR) for proteinuria may change when the urinary pyrogallol red (PR) protein assay method is changed to the benzethonium chloride method (BC). This study aimed to evaluate the difference between BC-based PCR (BC-PCR) and PR-based PCR (PR-PCR) values in children.

Methods: We compared the BC-PCR and PR-PCR values in the same first-morning urine samples without significant proteinuria in school urine screening settings. The upper limit of the reference values was set at the 97.5th percentile.

Results: Notably, 133 samples from 124 individuals (female: 62%, age: median 12.3 years, range 6.3-16.9 years) were collected between August 2020 and October 2022. The diagnoses included 34 normal individuals and 99 with asymptomatic hematuria. The urinary protein (UP) concentrations measured using the BC (BC-UP) and PR (PR-UP) methods were in a linear relationship; however, the BC-UP concentrations were higher than the PR-UP concentrations (mean of differences: 11.2, 95% confidence interval (CI): 11.0-13.4 mg/dL). Also, the BC-PCR values were higher than the PR-PCR values (mean of differences: 0.090, 95% CI: 0.082-0.098 g/gCr). The BC-PCR showed a body-size-related decrease, reflecting a body-size-related urinary creatinine increase. The suggested BC-PCR reference values for proteinuria were 0.25 and 0.17 g/gCr for elementary (6-12.4 years) and junior high school students (12.5-16 years), respectively. These values were higher than those of the PR-PCR and need further studies.

Conclusions: When evaluating PCR results, the urinary protein assay should be stated.

Background: Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by degeneration of lower motor neurons, resulting in progressive muscle weakness and atrophy. However, little is known regarding the cardiac function of children with SMA.

Methods: We recruited SMA patients younger than 18 years of age from January 1, 2022, to April 1, 2022, in the First Affiliated Hospital of Sun Yat-sen University. All patients underwent a comprehensive cardiac evaluation before treatment, including history taking, physical examination, blood tests of cardiac biomarkers, assessment of echocardiography and electrocardiogram. Age/gender-matched healthy volunteers were recruited as controls.

Results: A total of 36 SMA patients (26 with SMA type 2 and 10 with SMA type 3) and 40 controls were enrolled in the study. No patient was clinically diagnosed with heart failure. Blood tests showed elevated values of creatine kinase isoenzyme M and isoenzyme B (CK-MB) mass and high-sensitivity cardiac troponin T (hs-cTnT) in spinal muscular atrophy (SMA) patients. Regarding echocardiographic parameters, SMA children were detected with lower global left and right ventricular longitudinal strain, abnormal diastolic filling velocities of trans-mitral and trans-tricuspid flow. The results revealed no clinical heart dysfunction in SMA patients, but subclinical ventricular dysfunction was seen in SMA children including the diastolic function and myocardial performance. Some patients presented with elevated heart rate and abnormal echogenicity of aortic valve or wall. Among these SMA patients, seven patients (19.4%) had scoliosis. The Cobb's angles showed a significant negative correlation with LVEDd/BSA, but no correlation with other parameters, suggesting that mild scoliosis did not lead to significant cardiac dysfunction.

Conclusions: Our findings warrant increased attention to the cardiac status and highlight the need to investigate cardiac interventions in SMA children.

Background: Regular prophylaxis with activated prothrombin complex concentrates (aPCCs) is effective in adult patients with hemophilia with inhibitors; however, data in children are scarce.

Methods: This was a single-center retrospective study at Saitama Children's Medical Center. Patients with severe and moderate hemophilia with inhibitors aged <15 years at the start of aPCCs prophylaxis were included. Medical records were retrospectively reviewed.

Results: We treated nine pediatric patients with hemophilia with inhibitors (median age, 1.9 years; age range, 1.3-12.9 years; inhibitor titers before treatment with aPCCs, 5.9-69 BU/mL) using prophylactic aPCCs (doses, 50-100 U/kg; 2-3 times/week). The median prophylactic period was 13 months (range: 5-31 months). The median annualized bleeding rate (ABR) during prophylactic treatment with aPCCs was 2 (range, 0-17). In four patients, ABR was reduced by 19%-100% with prophylactic aPCCs compared to on-demand aPCCs. An adverse effect of treatment was that a patient with hemophilia B developed nephrotic syndrome 34 months after starting regular prophylaxis with aPCCs.

Conclusions: Regular prophylactic aPCCs reduced the ABR even in younger children with hemophilia A and B. Serious adverse events include nephrotic syndrome, which requires caution.

Background: Recent reports have demonstrated promising results regarding the use of ketamine sedation for reducing pediatric intussusception without an associated elevated risk of bowel perforation. However, data on direct intraluminal pressure are still lacking. This study aimed to investigate sedation safety, primarily by comparing intraluminal pressure.

Methods: This retrospective study included patients aged 10 years or younger, diagnosed with intussusception at a university-affiliated pediatric emergency department (ED) between July 2021 and February 2023. These patients were categorized into two groups: sedation and non-sedation. During regular working hours (from 9:00 a.m. to 5:00 p.m. on weekdays), patients were administered 1 mg/kg of intravenous ketamine for sedation during air enema reduction. Patients within non-working hours did not receive sedative interventions.

Results: In a study of 114 patients with intussusception (median age: 25 months), 29 (25.4%) received sedatives, and 85 (74.6%) did not. Maximum intraluminal pressure during the procedure showed no significant difference between the groups (sedation: 64 mmHg, non-sedation: 83 mmHg, p = 0.091). Bowel perforation was not observed in the overall cohort. No difference was observed in the failure rate or recurrence rate within 24 h between the two groups. Sedation with a median dose of 1 mg/kg ketamine did not cause delays in the ED process and demonstrated no adverse events while maintaining appropriate sedation depth with sequential dosing.

Conclusions: The utilization of ketamine sedation during fluoroscopy-guided air enema reduction for pediatric intussusception was not associated with increased intraluminal pressure, increased rate of reduction failure, or bowel perforation.

Background: The stigma associated with coronavirus disease 2019 (COVID-19) is a global problem that causes psychosomatic distress, including depression, anxiety, and loneliness. However, few studies have investigated the stigma of COVID-19 and the associated mental health impact on children or parents.

Methods: We conducted a prospective cohort study at the National Center for Child Health and Development in Tokyo, Japan, between November 2021 and October 2022. Children (4-17 years of age) with COVID-19 and parents of hospitalized children (0-17 years of age) with COVID-19 were enrolled in the study. Children with special health-care needs were excluded. The questionnaires on stigma and mental health (depression, anxiety, and loneliness) were administered during hospitalization and at the 1 month follow-up evaluation after discharge.

Results: During the study period, 47 children and 111 parents were included. Thirty-eight children (81%) and 105 parents (95%) answered the questionnaires at the 1 month follow up, respectively. Approximately 70% of participants were categorized as a high-stigma group. In children, subjective stigma was associated with loneliness during hospitalization (mean difference [MD] 2.32; 95% confidence interval [CI], 0.11-4.52) and depression at the 1 month follow up (MD 2.44; 95% CI, 0.40-4.48). In parents, presumed stigma was associated with depression, anxiety, and loneliness at 1 month follow up (MD 2.24, 1.68, and 1.15; 95% CI, 0.58-3.89, 0.11-3.25, and 0.08-2.21).

Conclusion: Our findings suggest that the stigma associated with COVID-19 continues to affect mental health for more than a month after discharge, and the effects of stigma on mental health differed between the children and parents.